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Clinical Outcomes of Diffuse Sclerosing Variant Papillary Thyroid Carcinoma in Pediatric Patients.

Authors
  • Brady, Charles1
  • Manning, Scott C2
  • Rudzinski, Erin3
  • Paulson, Vera3
  • Wang, Xing4
  • Liu, Yajuan J3
  • Parikh, Sanjay R2
  • Bonilla-Velez, Julianna2
  • Hawkins, Douglas S5
  • Dahl, John2
  • 1 UW Medicine, University of Washington School of Medicine, Seattle, Washington, U.S.A.
  • 2 Division of Pediatric Otolaryngology, Seattle Children's Hospital, Seattle, Washington, U.S.A.
  • 3 Department of Laboratory Medicine and Pathology, Seattle Children's Hospital, Seattle, Washington, U.S.A.
  • 4 Seattle Children's Research Division, Seattle Children's Hospital, Seattle, Washington, U.S.A.
  • 5 Division of Pediatrics, Seattle Children's Hospital, Seattle, Washington, U.S.A.
Type
Published Article
Journal
The Laryngoscope
Publisher
Wiley (John Wiley & Sons)
Publication Date
May 01, 2022
Volume
132
Issue
5
Pages
1132–1138
Identifiers
DOI: 10.1002/lary.29926
PMID: 34713899
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

The diffuse sclerosing variant of papillary thyroid carcinoma (DSV) may be more aggressive than conventional well-differentiated non-DSV related papillary thyroid carcinomas (N-PTC). Retrospective chart review. Retrospective review of clinical outcomes for patients 21 years of age or younger who underwent initial surgery for PTC at a single institution from January 1, 2005 to April 1, 2020. Genomic analysis was performed using targeted next-generation sequencing. Data were analyzed using Fischer's exact test and Kaplan-Meier curve log-rank test. Our cohort consisted of 72 patients, nine with DSV and 63 with N-PTC. Age at diagnosis was comparable (15.4 vs. 16.2 years, respectively, P = .46). DSV were more likely to be in the high-risk American Thyroid Academy pediatric risk group (100% vs. 41.3%, P = .004), to present with regional cervical lymph node metastases (100% vs. 60.3%, P = .036), and to present with distant metastases (67% vs. 22%, P = .005). No mortality seen in either group over 27.5 (interquartile range 14.8, 46.00) months average follow-up. Throughout the follow-up period, DSV were more likely to experience progression than N-PTC (hazard ratio = 5.7 [95% confidence interval 1.7-20.0; P = .0056]). In a subset of 19 patients with aggressive disease who had molecular testing as part of clinical care we detected RET fusions in nearly all DSV compared to a minority of N-PTC (83% vs. 15.4%, P = .0095). Pediatric patients with DSV have more advanced disease at diagnosis and are more likely to experience progression of disease compared to patients with N-PTC. The prevalence of RET fusions in our cohort recapitulates the frequency of this alteration described in prior studies. 4 Laryngoscope, 132:1132-1138, 2022. © 2021 The American Laryngological, Rhinological and Otological Society, Inc.

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